Anomalous origin of the right coronary artery (RCA) from the pulmonary artery, ARCAPA, is an extremely rare congenital heart disease. Only 200 cases were reported from 1885 to the present. Patients diagnosed with ARCAPA can be either asymptomatic or can experience symptoms, such as heart murmur, dyspnea, or angina, shortly after birth or around 40–60 years of life. Usually, those with isolated ARCAPA are diagnosed later in life compared to those who associate other structural cardiac defects. We report two cases of anomalous origin of the right coronary artery at the level of the pulmonary artery trunk (ARCAPA) that were diagnosed by invasive coronary angiography. Although asymptomatic, general recommendations suggest an early corrective intervention to prevent complications such as myocardial ischemia and cardiac dysfunction, which can lead to sudden cardiac death.
Acute type A aortic dissection (ATAAD) is a surgical emergency with a mortality of 1–2% per hour. Since its discovery over 200 years ago, surgical techniques for repairing a dissected aorta have evolved, and with the introduction of hypothermic circulatory arrest and cerebral perfusion, complex techniques for replacing the entire aortic arch were possible. However, postoperative neurological complications contribute significantly to mortality in this group of patients. The aim of this study was to determine the association between different bilateral selective antegrade cerebral perfusion (ACP) times and the incidence of postoperative ischemic stroke in patients with emergency surgery for ATAAD. Patients with documented hemorrhagic or ischemic stroke, clinical signs of stroke or neurological dysfunction prior to surgery, that died on the operating table or within 48 h after surgery, from whom the postoperative neurological status could not be assessed, and with incomplete medical records were excluded from this study. The diagnosis of postoperative stroke was made using head computed tomography imaging (CT) when clinical suspicion was raised by a neurologist in the immediate postoperative period. For selective bilateral antegrade cerebral perfusion, we used two balloon-tipped cannulas inserted under direct vision into the innominate artery and the left common carotid artery. Each cannula is connected to a separate pump with an independent pressure line. Near-infrared spectroscopy was used in all cases for cerebral oxygenation monitoring. The circulatory arrest was initiated after reaching a target core temperature of 25–28 °C. In total, 129 patients were included in this study. The incidence of postoperative ischemic stroke documented on a head CT was 24.8% (31 patients), and postoperative death was 20.9% (27 patients). The most common surgical technique performed was supravalvular ascending aorta and Hemiarch replacement with a Dacron graft in 69.8% (90 patients). The mean cardiopulmonary bypass time was 210 +/− 56.874 min, the mean aortic cross-clamp time was 114.775 +/− 34.602 min, and the mean cerebral perfusion time was 37.837 +/− 18.243 min. Using logistic regression, selective ACP of more than 40 min was independently associated with postoperative ischemic stroke (OR = 3.589; 95%CI = 1.418–9.085; p = 0.007). Considering the high incidence of postoperative stroke in our study population, we concluded that bilateral selective ACP should be used with caution, especially in patients with severely calcified ascending aorta and/or aortic arch and supra-aortic vessels. All efforts should be made to minimize the duration of circulatory arrest when using bilateral selective ACP with a target of less than 30 min, in hypothermia, at a body temperature of 25–28 °C.
Cunninghamella spp. is a group of fungi belonging to the Mucorales order. Cases of fungal endocarditis are sporadic, but more frequent in immunocompromised patients. COVID-19 (SARS-CoV-2 Infection Disease 2019) infections, prematurity, deferoxamine treatment, iron overload, neutropenia, diabetes, and malignant hemopathies proved to be risk factors for mucormycosis. We present the case of a 7-year-old boy who was treated every three weeks with blood transfusion for major beta-thalassemia, receiving deferoxamine for secondary hemochromatosis. After two weeks with nonspecific respiratory and digestive symptoms, he was admitted for fever, followed by lower limb ischemia and neurological signs. Echocardiography revealed massive endocarditis affecting the mitral and tricuspid valves with embolization phenomena in the brain, lungs, kidney, spleen, and lower limbs. As a particular finding, IgG antibodies for COVID-19 were positive. Emergency cardiac surgery was performed. The mitral valve necessitated replacement with CarboMedics prosthesis. Unfortunately, the patient did not survive. Cunninghamella spp. was confirmed via the PCR analysis of vegetations. Cunninghamella endocarditis in the context of a systemic infection presented as an opportunistic infection affecting a child who had several risk factors. Mucormycosis is challenging to treat, with high mortality. Prophylactic treatment in beta-thalassemia patients with iron-chelator deprivation drugs, such as deferiprone, may help in preventing these particular fungal infections.
Congenital heart disease (CHD) remains a predisposing cardiac condition for infective endocarditis (IE). Case report: We present the case of 8-year-old boy with no known pre-existing cardiac disease diagnosed with infective endocarditis (IE) with Gemella sanguinis. After admission, he underwent transthoracic echocardiography (TTE), which revealed the presence of Shone syndrome with a bicuspid valve, mitral parachute valve and severe aortic coarctation. He developed a paravalvular aortic abscess with severe aortic regurgitation and left ventricle (LV) systolic dysfunction for which he required a complex surgical intervention after six weeks of antibiotic treatment, consisting of Ross operation and coarctectomy, with a complicated postoperative course, cardiac arrest and ECMO support for five days. The evolution was slow and favorable, with no significant residual valvular lesions. However, persistent LV systolic dysfunction and increased muscle enzymes required further investigation to establish a genetic diagnosis of Duchenne disease. As Gemella is not considered a frequent pathogen of IE, no current guidelines refer specifically to it. Additionally, the predisposing cardiac condition of our patient is not currently classified as “high-risk” for IE; this is not considered an indication for IE prophylaxis in the current guidelines. Conclusion: This case illustrates the importance of accurate bacteriological diagnosis in infective endocarditis and poses concerns regarding the necessity of IE prophylaxis in “moderate risk” cardiac conditions such as congenital valvular heart disease, especially aortic valve malformations.
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